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Background
Cholesteatomas may be acquired or congenital. These are cysts enclosing keratin surrounded by a squamous epithelium. Over time, it may increase in size and destroy the surrounding delicate bones of the middle ear. Hearing loss, dizziness, and facial muscle paralysis are rare but can result from continued cholesteatoma growth. Acquired cases may arise from several pathways. Most postulate an initiating episode of otitis media leading to tympanic membrane necrosis and repair or a retraction pocket (an invagination of part of the tympanic membrane). This leads to loss of the normal control mechanisms leading to invasion of stratified squamous epithelium through the tympanic membrane and subsequent growth. These tumors are pearly white masses present in the upper and posterior part of the middle ear cleft.
Congenital cholesteatoma is much less common. It is hypothesized that they arise from epidermoid cell rests formed during the development of the middle ear. These appear as a white sphere in the anterosuperior part of the tympanic membrane. Larger lesions may lead to bony erosion.
Under the microscope, acquired and congenital cases are similar and resemble an epidermal inclusion cyst of the skin with a cyst lining of keratinizing stratified squamous epithelium surrounding keratinaceous debris. The squamous epithelium is characteristically thin and devoid of rete pegs. If the tumor extends into the ossicles, bony erosion and granulation tissue may be observed.
PATHOGENESIS CHARACTERIZATION Expression of Matrix-Degrading Cysteine Proteinase Cathepsin K in Cholesteatoma
Torsten Hansen, M.D., Ronald E. Unger, Ph.D., Andreas Gaumann, M.D., Inga Hundorf, M.D., Jan Maurer, M.D., C. James Kirkpatrick, M.D., Ph.D., D.Sc. and Jörg Kriegsmann, M.D., Ph.D.
Institute of Pathology (TH, REU, AG, IH, CJK, JK) and Department of Oto-Rhino-Laryngology (JM), Johannes Gutenberg-University, Mainz, Germany
Mod Pathol 2001;14:1226-1231 Abstract quote
Cholesteatoma is a nonneoplastic lesion of the middle ear space or mastoid that is histologically characterized by a progressive bone erosion of the ossicles and surrounding bone. Several matrix-degrading enzymes have been implicated as mediators of this bone erosion. Because the novel cysteine proteinase cathepsin K has been shown to play a central role in bone resorption, we examined the expression of this enzyme in tissue specimens of cholesteatoma.
Tissue specimens of 9 patients with cholesteatoma were obtained during middle-ear surgery. Expression of cathepsin K mRNA was determined by RT-PCR using specific primers. Immunohistochemical analysis of cathepsin K protein expression in tissue sections was performed by using the streptavidin-alkaline phosphatase technique. Expression of both cathepsin K mRNA and protein was detected in areas affected by cholesteatoma, whereas specimens of nonaffected ear cartilage and surrounding tissue were not positive. In addition, cathepsin K was detected in numerous multinucleated giant cells, particularly osteoclasts at the site of bone degradation. In contrast, keratinized squamous epithelium was negative for cathepsin K.
These data demonstrate that the matrix-degrading cysteine proteinase cathepsin K may be involved in bone erosion in cholesteatoma. Strong expression of this collagenolytic enzyme in osteoclasts suggests that these cells are mainly involved in cathepsin K-mediated bone destruction.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Squamous cell carcinoma Prominent rete pegs
Moderate or greater degree of dysplasia
PROGNOSIS AND TREATMENT CHARACTERIZATION Comparison of the incidence of cholesteatoma surgery before and after using ventilation tubes for secretory otitis media.
Rakover Y, Keywan K, Rosen G.
Department of Otorhinolaryngology, Ha'Emek Medical Center, Afula, Israel.
Int J Pediatr Otorhinolaryngol 2000 Nov 30;56(1):41-4 Abstrac quote
Inserting ventilation tubes has become, probably, the most common surgical operation performed on children in the free world. In our study, we attempted to examine whether the widely used procedure of inserting ventilation tubes in children with secretory otitis media is indeed justified in the long term.
For this purpose, we have studied the influence of the procedure on the formation of cholesteatoma by comparing the incidence of cholesteatoma surgery before and after using ventilation tubes in our department. The data were divided into two periods. The first period included the number of operations in the 10 years (1961-1970) before ventilation tubes were used as a routine treatment for secretory otitis media in our department. The second period included the number of operations in the last 10 years (1989-1998) following the usage of ventilation tubes over 30 years.
We found that the number of cholesteatoma operations has decreased, from 413 operations in the 10 years before ventilation tubes were used to 228 operations over the last 10 years. The number of operations per 10000 of the entire population has decreased from 20 operations to 6.6 operations after using ventilation tubes (P=0.0000001). We have shown that the incidence of surgery for cholesteatoma has been declining.
Having analyzed the options that might cause this decline, we believe that using ventilation tubes in secretory otitis media might help to reduce the incidence of cholesteatoma surgery in the long term.
Preference for the closed technique in the management of cholesteatoma of the middle ear in children: a retrospective study of 215 consecutive patients treated over 10 years.
Darrouzet V, Duclos JY, Portmann D, Bebear JP.
Ear, Nose and Throat Department, University Hospital of Bordeaux, France.
Am J Otol 2000 Jul;21(4):474-81 Abstract quote
OBJECTIVES: To evaluate clinical data, extensions, residual and recurrent lesion rates, and functional results in cases of cholesteatoma in pediatric patients.
DESIGN: A retrospective study at a single tertiary care center over a decade.
PATIENTS: In 199 children (mean age 9.6 years), 215 cholesteatomas were treated surgically, and the children were followed up for an average of 70 months (range 25-118 months).
INTERVENTIONS: Therapeutic and rehabilitative surgical procedures were done, using either a closed technique (CT) or an open technique: tympanoplasty in open technique (TOT), radical mastoidectomy (RM), or Rambo's technique)
MAIN OUTCOME MEASURES: Surgical findings, residual and recurrent lesions rate, and hearing assessment.
RESULTS: The first surgical procedure was CT in 88% of cases, TOT in 10%, RM and Rambo's technique in 1%. Two operations were done in 61.8% of children; 21% had three operations and 4.5% had four. Residual lesions were observed in 21.5% of all children, 32.8% in those undergoing a planned second look. Recurrence rate was 9.8%. Surprisingly, residual and recurrent lesion rates were higher after TOT (23.8% and 19%) than after CT (20.5%, 8.9%), but TOT-treated children had much more extensive lesions. A speech reception threshold (SRT) <30 dB hearing loss (HL) was achieved in 63.7% of patients, and normal hearing was obtained in 12.1%; SRT was significantly better after CT (mean 26.7 dB) than after TOT (mean 37.4 dB), after type II (mean 27 dB) than after type III (35 dB). A high-frequency pure-tone hearing loss >10 dB was uncommon (7.6%).
CONCLUSION: These results support the continued use of CT in most cases of cholesteatoma in children in developed countries
Postoperative results for cholesteatoma in children.
Mishiro Y, Sakagami M, Okumura S, Takeda N, Kubo T.
Department of Otolaryngology and Sensory Organ Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita City, Osaka, Japan.
Auris Nasus Larynx 2000 Jul;27(3):223-6 Abstract quote
OBJECTIVES: to review the postoperative results for cholesteatoma in children.
MATERIALS: 32 ears with acquired cholesteatoma in children operated on by a single surgeon between 1987 and 1995 and followed up more than 2 years. The mean follow-up period was 5.4 years.
RESULTS: a one-stage operation was performed in seven ears (21.9%) and preplanned stage operation in 25 ears (78.1%). In the first operation, closed tympanoplasty was performed in 31 ears (96.9%) and open tymapanoplasty in one ear (3.1%). During the second stage operation, residual cholesteatoma was found in 16 ears (64.0%). Recurrent cholesteatoma was detected in 19.4% of ears treated with closed tympanoplasty. The mean postoperative air conduction hearing level was within 20 dB in 12.5%, 30 dB in 40.6% and 40 dB in 78.1%. The hearing results of type III tympanoplasty was better than those of type IV tympanoplasty.
CONCLUSIONS: preplanned stage tympanoplasty is safer because of the high risk of recurrent and residual cholesteatoma. Surgical methods should be selected flexibly in individual cases depending upon cavity size, eustachian tube function and hearing level. Cholesteatoma in children should be operated on while stapes is present.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Last Updated 12/20/2001
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